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Autoimmunity
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
National Institutes of Health
Public Health Service • U.S. Department of Health and Human Services
For Your Information
This publication contains information about medications
used to treat the health condition discussed in this booklet.
When this booklet was printed, we included the most
up-to-date (accurate) information available. Occasionally,
new information on medication is released.
For updates and for any questions about any medications
you are taking, please contact the U.S. Food and Drug
Administration at 1–888–INFO–FDA (1–888–463–6332,
a toll-free call) or visit their Web site at www.fda.gov.
This booklet is not copyrighted. Readers are encouraged to
duplicate and distribute as many copies as needed.
Additional copies of this booklet are available from
National Institute of Arthritis and Musculoskeletal
and Skin Diseases
NIAMS/National Institutes of Health
1 AMS Circle
Bethesda, MD 20892–3675
You can also find this booklet on the NIAMS Web site at
www.niams.nih.gov/hi/topics/autoimmune/autoimmunity.htm.
Autoimmunity
Table of Contents
What Is Autoimmunity? . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
What Causes Autoimmunity? . . . . . . . . . . . . . . . . . . . . . . . . 1
What Kinds of Problems Are Caused by
Autoimmunity? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
How Are Autoimmune Diseases Diagnosed? . . . . . . . . . . . 5
How Are Autoimmune Diseases Treated? . . . . . . . . . . . . . . 6
What Types of Doctors Treat Autoimmune Diseases? . . . . 7
What Are Some Other Problems Related to
Autoimmune Diseases? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
What Research Is Being Conducted To Help People
With Autoimmune Diseases? . . . . . . . . . . . . . . . . . . . . . . . 11
Where Can People Find More Information About
Autoimmunity? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
Appendix I: Glossary of Terms . . . . . . . . . . . . . . . . . . . . . . 22
Appendix II: Glossary of Diseases . . . . . . . . . . . . . . . . . . . 24
iv
Autoimmunity
This booklet answers questions about autoimmunity. To
learn more about autoimmunity, your immune system, or
autoimmune diseases, speak with your doctor. You can also
check out the Appendices on pages 22–29.
What Is Autoimmunity?
When your body is attacked—perhaps by a virus or germs
on a nail you stepped on—your immune system defends
you. It sees and kills the germs that might hurt you.
But when the system doesn’t work right, this process can
cause harm. Immune cells can mistake your body’s own
cells as invaders and attack them. This “friendly fire”
can affect almost any part of the body. It can sometimes
affect many parts of the body at once. This is called
autoimmunity (meaning self-immunity).
What Causes Autoimmunity?
No one knows why the immune system treats some body
parts like germs. We do know that you can’t catch autoimmune diseases from another person.
Most scientists think that our genes and things in the environment are involved. If you have a certain gene or combination of genes, you may be at higher risk for autoimmune
disease. But you won’t get the disease until something
around you turns on your immune system. This may
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include the sun, infections, drugs, or, in some women,
pregnancy.
What Kinds of Problems Are Caused by
Autoimmunity?
Autoimmunity can affect almost any organ or body system.
The exact problems one has with autoimmunity (or its diseases) depends on which tissues are targeted.
If the skin is the target, you may have skin rashes, blisters,
or color changes. If it’s the thyroid gland, you may be tired,
gain weight, be more sensitive to cold, and have muscle
aches. If it’s the joints, you may have joint pain, stiffness,
and loss of function.
You may know which organ or system is affected from the
start. But you may not know the site of the attack. In many
people, the first symptoms are fatigue, muscle aches, and
low fever.
Where Does Autoimmunity Strike?
Because autoimmune diseases can affect almost any organ or
system of the body, one way to group them is by the body
system(s) they attack. The following is a list (not inclusive)
of body systems and the autoimmune diseases that can affect
them.
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Autoimmunity
Blood and blood vessels
• Autoimmune hemolytic anemia
• Pernicious anemia
• Polyarteritis nodosa
• Systemic lupus erythematosus
• Wegener’s granulomatosis
Digestive tract (including the mouth)
• Autoimmune hepatitis
• Behçet’s disease
• Crohn’s disease
• Primary bilary cirrhosis
• Scleroderma
• Ulcerative colitis
Eyes
• Sjögren’s syndrome
• Type 1 diabetes mellitus
• Uveitis
Glands
• Graves’ disease
• Thyroiditis
• Type 1 diabetes mellitus
Heart
• Myocarditis
• Rheumatic fever
• Scleroderma
• Systemic lupus erythematosus
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Joints
• Ankylosing spondylitis
• Rheumatoid arthritis
• Systemic lupus erythematosus
Kidneys
• Glomerulonephritis
• Systemic lupus erythematosus
• Type 1 diabetes mellitus
Lungs
• Rheumatoid arthritis
• Sarcoidosis
• Scleroderma
• Systemic lupus erythematosus
Muscles
• Dermatomyositis
• Myasthenia gravis
• Polymyositis
Nerves and brain
• Guillain-Barré syndrome
• Multiple sclerosis
• Systemic lupus erythematosus
Skin
• Alopecia areata
• Pemphigus/pemphigoid
• Psoriasis
• Scleroderma
• Systemic lupus erythematosus
• Vitiligo
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Autoimmunity
How Are Autoimmune Diseases Diagnosed?
Autoimmune diseases often don’t show a clear pattern of
symptoms at first. So diagnosing them can be hard. But
with time, a diagnosis can usually be made by using:
• Medical history—The doctor will ask about your
symptoms and how long you have had them. Your
symptoms may not point to one disease. But they
can be a starting point for your doctor. You should
tell your doctor if you have a family member with
autoimmune disease. You may not have the same
disease as your family member. But having a family
history of any autoimmune disease makes you more
likely to have one.
• Physical exam—During the exam, the doctor will
check for any signs. Inflamed joints, swollen lymph
nodes, or discolored skin might give clues.
• Medical tests—No one test will show that you have
an autoimmune disease. But doctors may find clues
in a blood sample. For example, people with lupus
or rheumatoid arthritis often have certain autoantibodies in their blood. Autoantibodies are blood proteins formed against the body’s own parts.
Not all people with these diseases have these autoantibodies. And some people without autoimmune
disease do have them. So blood tests alone may not
always help. But if a person has disease symptoms
5
and autoantibodies, the doctor can be more sure of
a diagnosis.
The key is patience. Your doctor may be able to diagnose
your condition quickly based on your history, exam, and test
results. But the process often takes time. It may take several
visits to find out exactly what’s wrong and the best way to
treat it.
How Are Autoimmune Diseases Treated?
Autoimmunity takes many forms. There are also many
treatments for it. Treatment depends on the type of disease,
how severe it is, and its symptoms. Generally, treatments
have one of three goals:
• Relieving symptoms—If your symptoms bother you,
your doctor may suggest treatments that give some
relief. Relieving symptoms may be as simple as taking a drug for pain relief. It may also be as involved
as having surgery.
• Preserving organ function—When autoimmune diseases threaten organs, treatment may be needed to
prevent damage. Such treatments may include drugs
to control an inflamed kidney in people with lupus.
Insulin injections can regulate blood sugar in people
with diabetes. These treatments don’t stop the disease. But they can save organ function. They can
also help people live with disease complications.
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Autoimmunity
• Targeting disease mechanisms—Some drugs may
also be used to target how the disease works. In
other words, they can suppress the immune system.
These drugs include cyclophosphamide (Cytoxan*)
and cyclosporine (Neoral and Sandimmune). The
same immune-suppressing drug may be used for
many diseases.
Your doctor may not prescribe a treatment. If your symptoms are mild, the risks of treatment may be worse than the
symptoms. You may choose to put off treatment for now.
But you should watch for signs that your disease is progressing. Visit your doctor regularly. You need to catch changes
before they lead to serious damage.
What Types of Doctors Treat Autoimmune
Diseases?
Treatments for autoimmune diseases vary. So do the types
of doctors who provide them.
For some people, one doctor will be enough to manage their
disease. Others may require a team approach. One doctor
might coordinate and give care, and others would treat specific organ problems. For example, a person with lupus
might be seen by a rheumatologist. But that person might
* Brand names included in this booklet are provided as examples only, and their
inclusion does not mean that these products are endorsed by the National Institutes
of Health or any other Government agency. Also, if a particular brand name is not
mentioned, this does not mean or imply that the product is unsatisfactory.
7
also see a nephrologist for related kidney problems and a
dermatologist for skin problems.
Specialists you may need to see include:
• A rheumatologist, who treats arthritis and other
rheumatic diseases. These include scleroderma and
systemic lupus erythematosus (lupus or SLE).
• An endocrinologist, who treats gland and hormone
problems. These include diabetes and thyroid
disease.
• A neurologist, who treats nerve problems. These
include multiple sclerosis and myasthenia gravis.
• A hematologist, who treats diseases that affect the
blood. These include pernicious anemia and
autoimmune hemolytic anemia.
• A gastroenterologist, who treats problems with the
digestive system. These include Crohn’s disease and
ulcerative colitis.
• A dermatologist, who treats problems of the skin,
hair, and nails. These include psoriasis, pemphigus/
pemphigoid, and alopecia areata.
• A nephrologist, who treats kidney problems. These
include glomerulonephritis, inflamed kidneys associated with lupus.
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Autoimmunity
What Are Some Other Problems Related to
Autoimmune Diseases?
Having a chronic disease can affect almost every part of your
life. The problems you might have with an autoimmune
disease vary. They may include:
• How you look and your self-esteem—Depending on
your disease, you may have discolored or damaged
skin or hair loss. Your joints may look different.
These can all affect how you look and your selfesteem. Such problems can’t always be prevented.
But their effects can be reduced with treatment.
Cosmetics, for example, can hide a skin rash.
Surgery can correct a malformed joint.
• Caring for yourself—Painful joints or weak muscles
can make it hard to do simple tasks. You may have
trouble climbing stairs, making your bed, or brushing
your hair. If doing daily tasks is hard, talk with a
physical therapist. The therapist can teach you exercises to improve strength and function. An occupational therapist can show you new ways to do things
or tools to make tasks easier. Sometimes regular
exercise or simple devices can help you do more
things on your own.
• Family relationships—Family members may not
understand why you don’t have energy to do things
you used to do. They may even think you are just
being lazy. But they may also be overly concerned
9
and eager to help you. They may not let you do the
things you can do. They may even give up their own
interests to be with you. Learn as much as you can
about your disease. Share what you learn with your
family. Involve them in counseling or a support
group. It may help them better understand the disease and how they can help.
• Sexual relations—Sexual relationships can also be
affected. For men, diseases that affect blood vessels
can lead to problems with erection. In women, damage to glands that produce moisture can lead to vaginal dryness. This makes intercourse painful. In both
men and women, pain, weakness, or stiff joints may
make it hard for them to move the way they once did.
They may not be sure about how they look. Or they
may be afraid that their partner will no longer find
them attractive. With communication, good medical
care, and perhaps counseling, many of these issues
can be overcome or at least worked around.
• Pregnancy and childbearing—In the past, women
with some autoimmune diseases were told not to
have children. But better treatments and understanding have changed that advice. Autoimmune
diseases can affect pregnancy, and pregnancy can
affect autoimmune diseases. But women with many
such diseases can safely have children. How a pregnancy turns out can vary by disease and disease
severity. If you have an autoimmune disease, you
should consult your doctor about having children.
10
Autoimmunity
What Research Is Being Conducted To Help People
With Autoimmune Diseases?
The National Institutes of Health (NIH) supports research
in autoimmune diseases. Here are a few examples:
• Rheumatoid arthritis—The National Institute of
Arthritis and Musculoskeletal and Skin Diseases
(NIAMS) and the North American Rheumatoid
Arthritis Consortium will study 1,000 siblings with
rheumatoid arthritis. Scientists will look at gene
material to find and identify parts of DNA involved
in the disease. They will test for proteins called
rheumatoid factor in the blood. And they will look
at x rays of the joints. This work will provide basic
facts about the genetics of the disease.
• Systemic lupus erythematosus (SLE)—NIAMSsupported scientists are studying whether women
with lupus can safely take oral contraceptives or
hormone replacement therapy. Previous research
suggests that female hormones may contribute to
the disease or make it worse.
• Lupus nephritis—One NIAMS project is testing a
drug that may be less toxic than the drugs now used
for lupus nephritis (kidney disease caused by lupus).
• Vitiligo—With NIAMS support, scientists are studying genes from pairs of siblings affected by this skin
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pigmentation disorder. They hope to find genes that
may cause vitiligo and learn how they affect the skin.
• Type 1 diabetes—Researchers supported by the
National Institute of Diabetes and Digestive and
Kidney Diseases have found a way to identify people
who are likely to get type 1 diabetes (formerly known
as juvenile diabetes). They are now testing ways to
prevent these people from getting the disease.
• Multiple sclerosis—Scientists supported by the
National Institute of Neurological Disorders and
Stroke are looking at the autoimmune system, infectious agents, and genes as culprits in multiple
sclerosis (MS). Such studies strengthen the theory
that MS comes from a number of factors rather than
a single one. Studies use magnetic resonance imaging to see how MS lesions evolve in the brain’s white
matter. Research has shown that MS has no bad
effects on pregnancy, labor, or delivery. In fact, the
stabilizing or remission of symptoms during pregnancy may be due to changes in a woman’s immune
system that allow her to carry a baby.
• Multiple autoimmune diseases—The National
Institute of Allergy and Infectious Diseases is supporting clinical trials of drugs that prevent the
immune system from attacking healthy cells. The
Institute wants to see if they are safe and useful.
Such drugs may prove helpful for treating a
number of autoimmune diseases.
12
Autoimmunity
Where Can People Find More Information About
Autoimmunity?
Various parts of the NIH provide information on different
aspects of autoimmune diseases. Many nonprofit organizations have patient resources, local chapters, and support
groups. Your own doctor can best tell you about your own
medical condition.
National Institutes of Health
■
National Institute of Arthritis and Musculoskeletal
and Skin Diseases (NIAMS)
1 AMS Circle
Bethesda, MD 20892–3675
Phone: 301–495–4484 or
877–22–NIAMS (226–4267) (free of charge)
TTY: 301–565–2966
Fax: 301–718–6366
www.niams.nih.gov
■
National Institute of Allergy and Infectious Diseases
Office of Communications
Building 31, Room 7A50
31 Center Drive, MSC 2520
Bethesda, MD 20892–2520
Phone: 301–496–5717
www.niaid.nih.gov/publications
and
www.niaid.nih.gov/clintrials/default.htm (for clinical
trials information)
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National Institute of Diabetes and Digestive and
Kidney Diseases (NIDDK)
National Diabetes Information Clearinghouse (NDIC)
1 Information Way
Bethesda, MD 20892–3560
Phone: 301–654–3327 or 800–860–8747 (free of charge)
E-mail: [email protected]
www.niddk.nih.gov
■
National Institute of Neurological Disorders and
Stroke
Office of Scientific and Health Reports
P.O. Box 5801
Bethesda, MD 20824
Phone: 301–496–5751 or 800–352–9424 (free of charge)
www.ninds.nih.gov
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NIH Clinical Center
Patient Recruitment and Public Liaison—for specific
NIH clinical trials information
Building 61
10 Cloister Court
Bethesda, MD 20892–4754
Phone: 800–411–1222 (free of charge)
TTY: 866–411–1010
E-mail: [email protected]
http://clinicalstudies.info.nih.gov/referring_patient.html
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Office of Rare Diseases
6100 Executive Boulevard
Room 3B01, MSC 7518
Bethesda, MD 20892–7518
Phone: 301–402–4336
http://rarediseases.info.nih.gov
Autoimmunity
Other Resources Sponsored by the U.S. Department of Health
and Human Services
■
Combined Health Information Database
http://chid.nih.gov
■
National Health Information Center
Phone: 301–565–4167 or 800–336–4797 (free of charge)
Health Finder: www.healthfinder.gov
Other Organizations
■
American Academy of Dermatology
P.O. Box 4014
Schaumburg, IL 60168–4014
Phone: 847–330–0230 or
888–462–DERM (3376) (free of charge)
Fax: 847–330–0050
www.aad.org
or
1350 Eye Street, N.W., Suite 880
Washington, DC 20005–4355
Phone: 202–842–3555
Fax: 202–842–4355
■
American Academy of Orthopaedic Surgeons
P.O. Box 2058
Des Plaines, IL 60017
Phone: 800–824–BONE (2663) (free of charge)
www.aaos.org
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American College of Rheumatology
1800 Century Place, Suite 250
Atlanta, GA 30345
Phone: 404–633–3777
Fax: 404–633–1870
E-mail: [email protected]
www.rheumatology.org
■
American Autoimmune Related Diseases Association
22100 Gratiot Avenue
East Detroit, MI 48021
Literature requests: 800–598–4668 (free of charge)
Phone: 586–776–3900
Fax: 586–776–3903
E-mail: [email protected]
www.aarda.org
■
American Behçet’s Disease Association
P.O. Box 19952
Amarillo, TX 79114
Phone: 800–7–BEHCET (723–4238) (free of charge)
www.behcets.com
■
American Diabetes Association
Attn: National Call Center
1701 N. Beauregard Street
Alexandria, VA 22311
Phone: 800–DIABETES (342–2383) (free of charge)
www.diabetes.org
Autoimmunity
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American Liver Foundation
75 Maiden Lane, Suite 603
New York, NY 10038
Phone: 800–GO–LIVER (465–4837) (free of charge) or
800–4HEP–USA (443–7872) (free of charge)
E-mail: [email protected]
www.liverfoundation.org
■
Arthritis Foundation
1330 West Peachtree Street, Suite 100
Atlanta, GA 30309
Phone: 404–872–7100 or 800–283–7800 (free of charge)
www.arthritis.org
■
Crohn’s and Colitis Foundation of America
National Headquarters
386 Park Avenue South, 17th Floor
New York, NY 10016–8804
Phone: 800–932–2423 (free of charge)
800–343–3637 (Warehouse)
www.ccfa.org
■
Guillain-Barré Syndrome Foundation International
P.O. Box 262
Wynnewood, PA 19096
Phone: 610–667–0131
Fax: 610–667–7036
E-mail: [email protected]
www.guillain-barre.com
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Juvenile Diabetes Research Foundation International
120 Wall Street
New York, NY 10005–4001
Phone: 212–785–9500 or
800–JDF–CURE (533–2873) (free of charge)
Fax: 212–785–9595
E-mail: [email protected]
www.jdf.org
■
Lupus Foundation of America, Inc.
2000 L Street, N.W., Suite 710
Washington, DC 20036
Phone: 202–349–1155 or 800–558–0121 (free of charge)
E-mail: [email protected]
www.lupus.org
■
Myasthenia Gravis Foundation of America
1821 University Avenue W., Suite S256
St. Paul, MN 55104
Phone: 651–917–6256 or 800–541–5454 (free of charge)
Fax: 651–917–1835
E-mail: [email protected]
www.myasthenia.org
■
The Myositis Association
1233 20th Street, N.W., Suite 402
Washington, DC, 20036
Phone: 202–887–0088
Fax: 202–466–8940
E-mail: [email protected]
www.myositis.org
Autoimmunity
n
National Adrenal Diseases Foundation
505 Northern Boulevard
Great Neck, NY 11021
Phone: 516–487–4992
E-mail: [email protected]
www.medhelp.org/nadf
n
National Alopecia Areata Foundation
P.O. Box 150760
San Rafael, CA 94915–0760
Phone: 415–472–3780
Fax: 415–472–5343
E-mail: [email protected]
www.naaf.org
n
National Multiple Sclerosis Society
733 Third Avenue, 6th Floor
New York, NY 10017–3288
Phone: 212–986–3240 or 800–344–4867 (free of charge)
Fax: 212–986–7981
E-mail: [email protected]
www.nmss.org
n
National Organization for Rare Diseases
55 Kenosia Avenue
P.O. Box 1968
Danbury, CT 06813-1968
Phone: 203–744–0100 or 800–999–6673 (free of charge)
TDD: 203–797–9590
E-mail: [email protected]
www.rarediseases.org
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International Pemphigus Foundation
1540 River Park Drive, Suite 208
Sacramento, CA 95815
Phone: 916–922–1298
Fax: 510–527–8497
E-mail: [email protected]
www.pemphigus.org
■
National Psoriasis Foundation
6600 SW 92nd Avenue, Suite 300
Portland, OR 97223–7195
Phone: 503–244–7404 or 800–723–9166 (free of charge)
Fax: 503–245–0626
E-mail: [email protected]
www.psoriasis.org
■
National Vitiligo Foundation
700 Olympic Plaza Circle, Suite 404
Tyler, TX 75701
Phone: 903–595–3713
Fax: 903–593–1545
E-mail: [email protected]
www.nvfi.org
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Scleroderma Foundation
12 Kent Way, Suite 101
Bayfield, MA 01922
Phone: 978–463–5843
Info line: 800–722–HOPE (4673) (free of charge)
Fax: 978–463–5809
E-mail: [email protected]
www.scleroderma.org
Autoimmunity
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Scleroderma Research Foundation
220 Montgomery Street, Suite 1411
San Francisco, CA 94104
Phone: 415–834–9444 or
800–441–CURE (2873) (free of charge)
Fax: 415–834–9177
E-mail: [email protected]
www.srfcure.org
■
Sjögren’s Syndrome Foundation
8120 Woodmont Avenue, Suite 530
Bethesda, MD 20814
Phone: 301–718–0300 or
800–4–SJOGRENS (475–6473) (free of charge)
Fax: 301–718–0322
E-mail: [email protected]
www.sjogrens.org or www.sjogrens.com
■
Spondylitis Association of America
P.O. Box 5872
Sherman Oaks, CA 91413
Phone: 800–777–8189 (free of charge)
E-mail: [email protected]
www.spondylitis.org
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S.L.E. Lupus Foundation
149 Madison Avenue, Suite 205
New York, NY 10016
Phone: 212–685–4118 or 800–74–LUPUS (745–8787)
(free of charge)
Fax: 212–545–1843
E-mail: [email protected]
www.lupusny.org
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Thyroid Foundation of America, Inc.
One Longfellow Place, Suite 1518
Boston, MA 02114
Phone: 800–832–8321 (free of charge)
Fax: 617–534–1515
E-mail: [email protected]
www.allthyroid.org
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Wegener’s Granulomatosis Association
P.O. Box 28660
Kansas City, MO 64188–8668
Phone: 800–277–9474 (free of charge)
Phone/Fax: 816–436–8211
E-mail: [email protected]
www.wgassociation.org
Appendix I: Glossary of Terms
Antibodies—Special proteins produced by the body’s
immune system that help fight and destroy viruses, bacteria,
and other foreign substances that invade the body.
Antigen—A substance (usually foreign) that stimulates the
immune response. In people with autoimmune disease,
the body’s own cells may be seen as antigens.
Autoantibodies—Abnormal antibodies that attack parts of
the body, causing autoimmune disease.
Autoimmune disease—A disease that occurs when the
immune system turns against parts of the body it is designed
to protect.
22
Autoimmunity
Fever—A rise in body temperature caused by the immune
system’s response to infection or disease.
Immune response—The reaction of the immune system
against foreign substances. When the reaction occurs
against the body’s own cells or tissues, it is called an autoimmune reaction.
Immune system—A complex system that normally protects
the body from infections. The immune system consists of a
group of cells, the chemicals that control those cells, and the
chemicals that those cells release.
Immunosuppressive drugs—Drugs that suppress the
immune response and can be used to treat autoimmune
disease. Unfortunately, because normal immunity is also
suppressed with these drugs, they leave the body at risk
for infection.
Infection—Invasion of the body tissues by bacteria or other
tiny organisms that cause illness.
Inflammation—A reaction of tissues to injury or disease,
typically marked by four signs: swelling, redness, heat,
and pain.
Trigger—Something that either sets off a disease in people
who are genetically predisposed to developing the disease, or
that causes a certain symptom to occur in a person who has
a disease. For example, sunlight can trigger rashes in people
with lupus.
23
Appendix II: Glossary of Diseases
Autoimmunity plays a role in more than 80 diseases. Following are brief descriptions of some of the many diseases
in which autoimmunity may be involved.
Note: Because the specific causes of many diseases are
unknown, there is debate among scientists about whether some
of these are truly autoimmune diseases. Your own doctor may
classify some of these diseases differently.
Alopecia areata—A disorder in which the immune system
attacks the hair follicles, causing loss of hair on the scalp,
face, and other parts of the body.
Ankylosing spondylitis—A rheumatic disease that causes
inflamed joints in the spine and sacroiliac (the joints that
connect the spine and the pelvis) and, in some people,
inflamed eyes and heart valves.
Arthritis—A general term for more than 100 different
diseases that affect the joints. Many forms of arthritis and
related conditions are believed to have an autoimmune
component.
Autoimmune hemolytic anemia—A condition in which
immune system proteins attack the red blood cells, resulting
in fewer of these oxygen-transporting cells.
24
Autoimmunity
Autoimmune hepatitis—A disease in which the body’s
immune system attacks liver cells, causing inflammation.
If not stopped, inflammation can lead to cirrhosis (scarring
and hardening) of the liver and eventually liver failure.
Behçet’s disease—A condition characterized by sores in the
mouth and on the genitals and by inflammation in parts of
the eye. In some people, the disease also results in inflammation of the joints, digestive tract, brain, and spinal cord.
Crohn’s disease—An inflammatory disease of the small
intestine or colon that causes diarrhea, cramps, and excessive
weight loss.
Dermatomyositis—A rare autoimmune disease that causes
patchy red rashes around the knuckles, eyes, and other parts
of the body along with chronic inflammation of the muscles.
It may occur along with other autoimmune diseases such as
rheumatoid arthritis or systemic lupus erythematosus.
Diabetes mellitus, type 1—A condition in which the
immune system destroys insulin-producing cells of the
pancreas, making it impossible for the body to use glucose
(blood sugar) for energy. Type 1 diabetes usually occurs in
children and young adults.
Glomerulonephritis—Inflammation of the kidney’s tiny filtering units, which in severe cases can lead to kidney failure.
25
Graves’ disease—An autoimmune disease of the thyroid
gland that results in the overproduction of thyroid hormone.
This causes such symptoms as nervousness, heat intolerance, heart palpitations, and unexplained weight loss.
Guillain-Barré syndrome—A disorder in which the body’s
immune system attacks part of the nervous system, leading
to numb, weak limbs and, in severe cases, paralysis.
Inflammatory bowel disease—The general name for diseases that cause inflammation in the intestine, the most
common of which are ulcerative colitis and Crohn’s disease.
Lupus nephritis—Damaging inflammation of the kidneys
that can occur in people with lupus. If not controlled, it
may lead to total kidney failure.
Multiple sclerosis—A disease in which the immune system
attacks the protective coating called myelin around the
nerves. The damage affects the brain and/or spinal cord and
interferes with the nerve pathways, causing muscular weakness, loss of coordination, and visual and speech problems.
Myasthenia gravis—A disease in which the immune system
attacks the nerves and muscles in the neck, causing weakness and problems with seeing, chewing, and/or talking.
Myocarditis—Inflamed and degenerating muscle tissue of
the heart that can cause chest pain and shortness of breath.
This can lead to congestive heart failure.
26
Autoimmunity
Pemphigus/pemphigoid—An autoimmune disease of the
skin characterized by itching and blisters.
Pernicious anemia—A deficiency of the oxygen-carrying red
blood cells that often occurs in people with autoimmune diseases of the thyroid gland.
Polyarteritis nodosa—An autoimmune disease that causes
inflammation of the small and medium-sized arteries. This
leads to problems in the muscles, joints, intestines, nerves,
kidney, and skin.
Polymyositis—A rare autoimmune disease characterized by
inflamed and tender muscles throughout the body, particularly those of the shoulder and hip girdles.
Primary biliary cirrhosis—A disease that slowly destroys
the bile ducts in the liver. When the ducts are damaged, bile
(a substance that helps digest fat) builds up in the liver and
damages liver tissue.
Psoriasis—A chronic skin disease that occurs when cells in
the outer layer of the skin reproduce faster than normal and
pile up on the skin’s surface. This results in scaling and
inflammation. An estimated 10 to 30 percent of people with
psoriasis develop an associated arthritis called psoriatic
arthritis.
27
Rheumatic fever—A disease that can occur following
untreated streptococcus (strep) infection. It most often
affects children, causing painful, inflamed joints and, in
some cases, permanent damage to heart valves.
Rheumatoid arthritis—A disease in which the immune
system is believed to attack the linings of the joints. This
results in joint pain, stiffness, swelling, and destruction.
Sarcoidosis—A disease characterized by granulomas (small
growths of blood vessels, cells, and connective tissue) that
can lead to problems in the skin, lungs, eyes, joints, and
muscles.
Scleroderma—An autoimmune disease characterized by
abnormal growth of connective tissue in the skin and blood
vessels. In more severe forms, connective tissue can build up
in the kidneys, lungs, heart, and gastrointestinal tract, leading in some cases to organ failure.
Sjögren’s syndrome—A condition in which the immune system targets the body’s moisture-producing glands, leading to
dryness of the eyes, mouth, and other body tissues.
Systemic lupus erythematosus—An autoimmune disease,
primarily of young women, that can affect many parts of the
body, including the joints, skin, kidneys, heart, lungs, blood
vessels, and brain.
28
Autoimmunity
Thyroiditis—An inflammation of the thyroid gland that
causes the gland to become underactive. This results in
symptoms such as fatigue, weakness, weight gain, cold intolerance, and muscle aches.
Ulcerative colitis—A disease that causes ulcers in the top
layers of the lining of the large intestine. This leads to
abdominal pain and diarrhea.
Uveitis—The inflammation of structures of the inner eye,
including the iris (the colored tissue that holds the lens of
the eye) and the choroid plexus (a network of blood vessels
around the eyeball). Uveitis occurs with some rheumatic
diseases, including ankylosing spondylitis and juvenile
rheumatoid arthritis.
Vitiligo—A disorder in which the immune system destroys
pigment-making cells called melanocytes. This results in
white patches of skin on different parts of the body.
Wegener’s granulomatosis—An autoimmune disease
that damages the small and medium-sized blood vessels
throughout the body, resulting in disease in the lungs,
upper respiratory tract, and kidneys.
29
Acknowledgments
The NIAMS gratefully acknowledges the assistance of Jill Buyon, M.D., of New
York University Medical Center, New York, NY; Elaine Collier, M.D., of the
National Institute of Allergy and Infectious Diseases, NIH; Elizabeth Gretz, M.D.,
of NIAMS, NIH; Peter Lipsky, M.D., of NIAMS, NIH; Michael Lockshin, M.D.,
of the Hospital for Special Surgery, New York, NY; Barbara Mittleman, M.D., of
NIAMS, NIH; Alan Moshell, M.D., of NIAMS, NIH; and Noel Rose, M.D.,
Ph.D., of the Johns Hopkins University School of Hygiene and Public Health,
Baltimore, MD, in the preparation and review of this booklet. Mary Anne Dunkin
was the primary author of this booklet.
30
The mission of the National Institute of Arthritis and
Musculoskeletal and Skin Diseases (NIAMS), a part of the
Department of Health and Human Services’ National Institutes of Health (NIH), is to support research into the causes,
treatment, and prevention of arthritis and musculoskeletal
and skin diseases, the training of basic and clinical scientists to
carry out this research, and the dissemination of information
on research progress in these diseases. The National Institute
of Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse is a public service sponsored by the
NIAMS that provides health information and information
sources. Additional information can be found on the NIAMS
Web site at www.niams.nih.gov.
U.S. Department of Health and Human Services
Public Health Service
National Institutes of Health
National Institute of Arthritis and
Musculoskeletal and Skin Diseases
NIH Publication No. 02– 4858
January 2002